Hard X-Ray flux upper limits of central compact objects in supernova remnants

We searched for hard X-ray (20–300 keV) emission from nine central compact objects (CCOs) 1E 1207.4−5209, 1WGA J1713−3949, J082157.5−430017, J085201.4−461753, J1601−5133, J1613483−5055, J181852.0−150213, J185238.6+004020, and J232327.9+584843 with the INTEGRAL observatory. We applied spectral imaging analysis and did not detect any of the sources with luminosity upper limits in the range of 1033-1034 ergs/s in the 20-75 keV band. For nearby CCOs (< 4 kpc) the upper limit luminosities are an order of magnitude lower than the measured persistent hard X-ray luminosities of AXPs. This may indicate that the central compact objects are low magnetic field systems with fallback disks around them

Hard X-Ray flux upper limits of central compact objects in supernova remnants

We searched for hard X-ray (20–300 keV) emission from nine central compact objects (CCOs) 1E 1207.4−5209, 1WGA J1713−3949, J082157.5−430017, J085201.4−461753, J1601−5133, J1613483−5055, J181852.0−150213, J185238.6+004020, and J232327.9+584843 with the INTEGRAL observatory. We applied spectral imaging analysis and did not detect any of the sources with luminosity upper limits in the range of 1033-1034 ergs/s in the 20-75 keV band. For nearby CCOs (< 4 kpc) the upper limit luminosities are an order of magnitude lower than the measured persistent hard X-ray luminosities of AXPs. This may indicate that the central compact objects are low magnetic field systems with fallback disks around them

Comparative effectiveness of drugs used to constrict the patent ductus arteriosus: a secondary analysis of the PDA-TOLERATE trial (NCT01958320).

ObjectiveTo evaluate the effectiveness of drugs used to constrict patent ductus arteriosus (PDA) in newborns &lt; 28 weeks.MethodsWe performed a secondary analysis of the multi-center PDA-TOLERATE trial (NCT01958320). Infants with moderate-to-large PDAs were randomized 1:1 at 8.1 ± 2.1 days to either Drug treatment (n = 104) or Conservative management (n = 98). Drug treatments were assigned by center rather than within center (acetaminophen: 5 centers, 27 infants; ibuprofen: 7 centers, 38 infants; indomethacin: 7 centers, 39 infants).ResultsIndomethacin produced the greatest constriction (compared with spontaneous constriction during Conservative management): RR (95% CI) = 3.21 (2.05-5.01)), followed by ibuprofen = 2.03 (1.05-3.91), and acetaminophen = 1.33 (0.55-3.24). The initial rate of acetaminophen-induced constriction was 27%. Infants with persistent moderate-to-large PDA after acetaminophen were treated with indomethacin. The final rate of constriction after acetaminophen ± indomethacin was 60% (similar to the rate in infants receiving indomethacin-alone (62%)).ConclusionIndomethacin was more effective than acetaminophen in producing ductus constriction

A Patient with Proopiomelanocortin Deficiency: An Increasingly Important Diagnosis to Make

Proopiomelanocortin (POMC) deficiency is a rare monogenic disorder with early-onset obesity. Investigation of this entity have increased our insight into the important role of the leptin-melanocortin pathway in energy balance. Here, we present a patient with POMC deficiency due to a homozygous c.206delC mutation in the POMC gene. We discuss the pathogenesis of this condition with emphasis on the crosstalk between hypothalamic and peripheral signals in the development of obesity and the POMC-melanocortin 4 receptors system as a target for therapeutic intervention

A national survey on use of less invasive surfactant administration in Turkey

Background. The aim of the study was to assess the rate of utilization, policy of premedication, technique, equipment, experience on safety and efficacy for less invasive surfactant administration or minimally invasive surfactant therapy (LISA/MIST) use in Turkey. Methods. An online survey was designed and distributed via Google Forms tool to 350 neonatologists from 173 units through NICU-Turk mailing list of the Turkish Neonatal Society. Participants were asked to answer the survey for their own neonatal intensive care unit (NICU). Results. LISA/MIST use rate was 81.6% among 87 NICUs which responded (response rate was 50.2%). LISA was used regularly in 23 of the units (26.4%), occasionally in 35 (40.2%), rarely in 12 (13.8%), and only for clinical trials in 1 (1.1%). LISA/MIST has been never applied in 16 units (18.4%). Conclusions. LISA/MIST is widely used in Turkey similar to several regions in Europe but unlike the USA. Future studies are expected to further clarify some questions about LISA/MIST procedure, especially on its efficacy and safety

Rare Types of Congenital Adrenal Hyperplasias Other Than 21-hydroxylase Deficiency

Although the most common cause of congenital adrenal hyperplasia (CAH) worldwide is 21-hydroxylase deficiency (21-OHD), which accounts for more than 95% of cases, other rare causes of CAH such as 11-beta-hydroxylase deficiency (11&#946;-OHD), 3-beta-hydroxy steroid dehydrogenase (3&#946;-HSD) deficiency, 17-hydroxylase deficiency and lipoid CAH (LCAH) may also be encountered in clinical practice. 11&#946;-OHD is the most common type of CAH after 21-OHD, and CYP11B1 deficiency in adrenal steroidogenesis causes the inability to produce cortisol and aldosterone and the excessive production of adrenal androgens. Although the clinical and laboratory features are similar to 21-OHD, findings of mineralocorticoid deficiency are not observed. 3&#946;-HSD deficiency, with an incidence of less than 1/1,000,000 live births, is characterized by impairment of both adrenal and gonadal steroid biosynthesis very early in life, with inadequate virilization in boys and varying degrees of virilization in girls. It may present with salt wasting crisis or delayed puberty in both genders. While 46,XY disorders of sex development is frequently observed in boys with 17-hydroxylase deficiency, immature pubertal development and primary amenorrhea are observed in girls due to estrogen deficiency throughout adolescence. Patients with LCAH, which develops due to steroidogenic acute regulatory protein deficiency, typically present with salt wasting in the first year of life. It is characterized by complete or near-complete deficiency of adrenal and gonadal steroid hormones and progressive accumulation of cholesterol esters in the adrenal gland

Two Siblings with Isolated GH Deficiency Due to Loss−of−Function Mutation in the GHRHR Gene: Successful Treatment with Growth Hormone Despite Late Admission and Severe Growth Retardation

Patients with growth hormone releasing hormone receptor (GHRHR) mutations exhibit pronounced dwarfism and are phenotypically and biochemically indistinguishable from other forms of isolated growth hormone deficiency (IGHD). We presented here two siblings with clinical findings of IGHD due to a nonsense mutation in the GHRHR gene who reached their target height in spite of late GH treatment. Two female siblings were admitted to our clinic with severe short stature at the age of 13.8 (patient 1) and 14.8 years (patient 2). On admission, height in patient 1 was 107 cm (−8.6 SD) and 117 cm (−6.7 SD) in patient 2. Bone age was delayed in both patients (6 years and 9 years). Clinical and biochemical analyses revealed a diagnosis of complete IGHD (peak GH levels on stimulation test was 0.06 ng/mL in patient 1 and 0.16 ng/mL in patient 2). Patients were given recombinant human GH treatment. Genetic analysis of the GH and GHRHR genes revealed that both patientscarried the GHRHR gene mutation p.Glu72X (c.214 G>T) in exon 3 in homozygous (or hemizygous) state. After seven years of GH treatment, the patients reached a final height appropriate for their target height. Final height was 151 cm (−1.5 SD) in patient 1 and 153 cm (−1.2 SD) in patient 2. In conclusion, genetic analysis is indicated in IGHD patients with severe growth failure and a positive family history. In spite of the very late diagnosis in these two patients who presented with severe growth deficit due to homozygous loss−of−function mutations in GHRHR, their final heights reached the target height

Efficacy and safety of Saccharomyces boulardii in amebiasis-associated diarrhea in children

The efficacy and safety of adding Saccharomyces boulardii to antibiotic treatment for amebiasis-associated acute diarrhea in children were assessed in this study. Forty-five children in Group I received only metronidazole per oral for 10 days while 40 patients in Group II received S. boulardii in addition to the same medication. The major outcomes investigated were duration of acute and bloody diarrhea, frequency and consistency of stools, resolution time of the symptoms, and the tolerance and side effects of the treatment regimens. The median duration of acute diarrhea was 5 (1-10) days in Group I and 4.5 (1-10) days in Group II (p=0.965). The median number of stools on follow-up and duration of bloody diarrhea, fever, abdominal pain and vomiting were similar in the two groups. S. boulardii was well tolerated by the children and no side effects were recorded. Addition of S. boulardii to antibiotic treatment of amebiasis-associated acute diarrhea in children does not seem to be more effective than metronidazole treatment alone

An observational, multicenter, registry-based cohort study of Turkish Neonatal Society in neonates with Hypoxic ischemic encephalopathy

BACKGROUND: Hypoxic ischemic encephalopathy (HIE) is a significant cause of mortality and short- and long-term morbidities. Therapeutic hypothermia (TH) has been shown to be the standard care for HIE of infants ≥36 weeks gestational age (GA), as it has been demonstrated to reduce the rates of mortality, and adverse neurodevelopmental outcomes. This study aims to determine the incidence of HIE in our country, to assess the TH management in infants with HIE, and present short-term outcomes of these infants. METHODS: The Turkish Hypoxic Ischemic Encephalopathy Online Registry database was established for this multicenter, prospective, observational, nationally-based cohort study to evaluate the data of infants born at ≥34 weeks GA who displayed evidence of neonatal encephalopathy (NE) between March, 2020 and April 2022. RESULTS: The incidence of HIE among infants born at ≥36 weeks GA (n = 965) was 2.13 per 1000 live births (517:242440), and accounting for 1.55% (965:62062) of all neonatal intensive care unit admissions. The rates of mild, moderate and severe HİE were 25.5% (n = 246), 58.9% (n = 568), and 15.6% (n = 151), respectively. Infants with severe HIE had higher rates of abnormal magnetic resonance imaging (MRI) findings, and mortality (p0.05). TH was administered to 85 (34.5%) infants with mild HIE, and of those born of 34-35 weeks of GA, 67.4% (n = 31) received TH. A total of 58 (6%) deaths were reported with a higher mortality rate in infants born at 34-35 weeks of GA (OR 3.941, 95% Cl 1.446-10.7422, p = 0.007). CONCLUSION: The incidence of HIE remained similar over time with a reduction in mortality rate. The timing of TH initiation, whether [removed